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FEBS Lett. 2013 Jul 11;587(14):2199-204. doi: 10.1016/j.febslet.2013.05.042. Epub 2013 May 24.

Scavenger receptor B1 post-translational modifications in Rett syndrome.

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  • 1Department of Life Sciences and Biotechnology, University of Ferrara, Ferrara, Italy.


The modulation of the HDL receptor scavenger receptor B1 (SRB1) was evaluated in skin fibroblasts isolated from patients with Rett syndrome (RTT), a genetic form of infantile autism. Patients showed an altered plasma lipid profile, while their skin fibroblasts showed a dramatic reduction in SRB1 (immunogold, Western blot and immunohistochemistry). The decreased SRB1 levels were demonstrated to be the consequence of its binding with 4-hydroxy-2-nonenal (4HNE), a product of lipid peroxidation, and its increased ubiquitination. Our findings show for the first time a loss of SRB1 in RTT cells and its relationship with a chronic oxidative stress status.

Copyright © 2013 Federation of European Biochemical Societies. Published by Elsevier B.V. All rights reserved.

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